[关键词]
[摘要]
成人眼眶黄色肉芽肿病是一种较罕见的眼眶和眼附属器疾病，属于非朗格汉斯组织细胞增多病(Ⅱ型)，分为四个亚型：成人起病的黄色肉芽肿、坏死性黄色肉芽肿、Erdheim-Chester病和成人起病的眶周黄色肉芽肿合并哮喘型。该病的诊断依靠临床表现和病理特征。治疗多为经验性，根据临床分型和表现的不同选择合适的治疗策略，主要为糖皮质激素、免疫抑制剂和手术治疗等。本文就该病的发病机制、临床分型、诊断和治疗的研究进展进行综述，以期为该病的临床诊治提供参考。
[Key word]
[Abstract]
Adult orbital xanthogranulomatous disease is a group of rare orbital and ocular adnexal disorders, which is classified as class II non-Langerhans histiocytic proliferations. This disease can be classified into 4 subtypes based mainly on systemic involvement: adult-onset xanthogranuloma, necrobiotic xanthogranuloma, Erdheim-Chester disease and adult-onset asthma and periocular xanthogranuloma. Diagnosis depends on its characteristic clinical manifestations and pathologic features. Therapeutic approaches are derived from anecdotal evidences, which include corticosteroid, immunosuppressive agent, surgical debulking and chemotherapy. The management of this disease varies with different subtypes, as well as associated systemic presentations. This review summarizes advances of etiopathogenesis, clinical features, diagnosis and treatment.
[中图分类号]
[基金项目]
上海市科委自然基金(No.16ZR1419600)
